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MEDICINE > 11 - Muscle and Soft Tissue Rheumatology > Flashcards

11 - Muscle and Soft Tissue Rheumatology Flashcards

1
Q

What are some autoimmune connective tissue disorders?

A
  • SLE
  • Systemic sclerosis
  • Sjogren’s
  • Polymyositis and Dermatomyositis
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2
Q

If someone has a multisystem disorder with raised ESR but normal CRP, what should you consider as the diagnosis?

A

SLE

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3
Q

What is the general pathophysiology of SLE?

A

Multisystemic autoimmune disease that relapses and remits

Autoantibodies made against autoantigens which then form immune complexes

- Inadequate clearance of immune complexes cause immune responses in tissues, leading to inflammation and tissue damage

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4
Q

What are some risk factors for developing SLE?

A

- Female

  • Child-bearing age

- Afro-Caribbean or Asian

- 1st or 2nd degree relative

  • UV light
  • Drugs
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5
Q

What are some common symptoms and signs of lupus?

A

Remitting and relapsing disease that often has non specific features like malaise, fatigue, myalgia and fever

Other features: alopecia, weight loss, lymphadenopathy, Raynaud’s

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6
Q

What investigations can you do if you suspect lupus and what will they show?

A

- ESR/PV: raised

- CRP: normal

- FBC: anaemia and leukopenia

- Autoantibodies: ANA, anti-dsDNA, anti-Ro, anti-La, antiphospholipid antibodies

- C3/C4: decreased in active lupus

- Urinalysis: may have haematuria if renal disease

- Biopsy: skin and renal, can be diagnostic

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7
Q

If a patient with lupus has antiphospholipid antibodies, what can this increase the chance of?

A
  • Pregnancy loss
  • Thrombosis

HIGH CVD RISK IN SLE REGARDLESS

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8
Q

How is SLE diagnosed?

A

SLE can mimic other diseases. Take skin/renal biopsies

Need 4 or more criteria (with at least 1 clinical and 1 labatory)

OR

Biopsy proven lupus nephritis with positive ANA or anti-dsDNA

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9
Q

How can we monitor SLE activity to monitor for flares?

A

- Anti-dsDNA antibody titres

- C3/C4 levels (will be low in flares as consumption)

- ESR

- Urine for protein and erythrocyte casts

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10
Q

What are some drugs that cause drug-induced lupus?

A
    • Isoniazid*
    • TNF-a inhibitors*
  • Chlorpromazine
  • Phenytoin
    • Hydralazine*
  • Procainamide
  • Minocycline

Stop drug for resolution

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11
Q

What are some drugs that can worsen idiopathic SLE?

A
  • Oral contraceptives
  • Sulfonamides
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12
Q

What is the management for lupus? (include general measure, maintenance and flares)

A

General measures: high factor sun-block, advice on healthy lifestyle due to CVD risk, topical steroids for skin flares, screen for co-morbidities and drug toxicity

Maintenance: Hydroxychloroquine for rash and arthralgia. NSAIDs if no renal disease. Mycophenolate mofetil, Azathioprine, Rituximab are used for steroid sparing

Flares: short course of low-dose steroids such as prednisolone, if organ damage use DMARDs or mycophenolate

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13
Q

How is lupus nephritis treated differently to lupus?

A

Intesive immunosuppression to induce remission using steroids and cyclophosphamide before maintenance

BP control needed.

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14
Q

What is antiphospholipid syndrome?

A

Antiphospholipid antibodies, often associated with SLE

Coagulation defect

Livedo reticularis

Obstetric (recurrent miscarriages)

Thrombocytopenia

Antiphospholipid syndrome (APS) is an autoimmune disorder that increases your risk of blood clots. It occurs when your immune system mistakenly produces antibodies that attack proteins called phospholipids, which are normally found in the cell membranes throughout your body. These antibodies can interfere with the blood clotting process, making it more likely for clots to form in arteries and veins.

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15
Q

What is Raynaud’s phenomenon?

A

- Peripheral digital ischaemia due to vasospasm.

- Painful and sequence of colour changes that are bilateral over few minutes

  • Usually precipitated by cold and emotion
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16
Q

What is Raynaud’s syndrome?

A

When Raynaud’s phenomenon is idiopathic.

Common in young women. Tell them to keep warm and avoid smoking

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17
Q

What are some causes of Raynaud’s?

A

- SLE

  • Scleroderma
  • Dermatomyositis and polymyositis

- Sjogren’s

- Drug induced by beta blockers

  • Heavy vibrating tools
  • Cervical rib
  • Sticky blood
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18
Q

How is Raynaud’s treated?

A

Conservative:

  • Stop smoking
  • Keep warm e.g gloves, handwarmers

Pharmacological:

- CCB’s like nifedipine 1st line.

  • PDE-5 inhibitors like sildenafil and epoprostenol also good
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19
Q

How does the pattern of Raynaud’s vary between idiopathic and secondary causes?

A

Idiopathic: last minutes and bilateral

Secondary: can last hours and can be asymmetrical with only a few digits involved

20
Q

What are some complications of Raynaud’s?

A
  • Digital ulcers
  • Infection
  • Gangrene
21
Q

What is nail fold capillaroscopy?

A

Non-invasive way of looking at structural changes in peripheral microcirculation.

Helps with diagnosis of scleroderma and DM with Raynaud’s

22
Q

What is vasculitis?

A

Inflammatory blood vessel disorder that gets clinical features from damage of blood vessel walls with subsequent thrombosis, ischaemia, bleeding and aneurysm formation

23
Q

How is a diagnosis of vasculitis made?

A
  • History using glove sweater trunk approach
  • Physical exam
  • Urine dipstick to check for glomerulonephritis
  • Tests to exclude other diagnoses
24
Q

How is vasculitis categorised, and give some examples in each category?

A

Size of blood vessel

Small: MPA, Wegener’s granulomatosis, IgA vasculitis, Goodpasture’s

Medium: Kawasaki disease, polyarteritis nodosa

Large: GCA, Takayasu’s arteritis

25
Q

How is vasculitis managed?

A
  • Rule out infection and stop any drugs in secondary causes
  • Large: corticosteroids

- Medium/Small: immunosuppresion with biological agents or immunosuppressants

26
Q

What is dermatomyositis and polymyositis?

A
  • Autoimmune muscle diseases that have symmetrical inflammation of striated muscle leading to muscle weakness
  • Can present with shortness of breath due to muscle weakness
  • Also may have fever, arthralgia, Raynaud’s, interstitial lung fibrosis and myocardial involvement
27
Q

How does dermatomyositis present differently to polymyositis?

A

Myositis plus skin signs that are photosensitive:

  • Macular rash (shawl sign)
  • Heliotrope rash on eyelids
  • Nailfold erythema
  • Gottron’s papules (knuckles, elbows and knees)
28
Q

What is the diagnostic criteria for dermatomyositis and polymyositis?

A
  • Myositis can be demonstrated on MRI
  • Raised CK
29
Q

How is dermatomyositis and polymyositis treated?

A

- High dose corticosteroids (Prednisolone) initially

- Methotrexate or Azathioprine long term

- IVIG

  • Sun protection
  • Hydroxychloroquine can help rash
30
Q

What is systemic sclerosis and the pathophysiology of this?

A

Multisystemic autoimmune disease that has:

- Scleroderma (skin fibrosis)

- Internal organ fibrosis

- Microvascular abnormalities

Due to increased fibroblast activity

Systemic sclerosis (SSc), also known as scleroderma, is a rare autoimmune disease that affects the connective tissues throughout the body. Connective tissues provide structure and support for the skin, organs, and other body parts. When the immune system mistakenly attacks these tissues, it causes scarring and thickening (fibrosis), leading to various symptoms and potential organ damage.

31
Q

What are the two types of scleroderma and how do they present?

A

Limited/CREST syndrome

  • More common
  • Face, hands and feet

Diffuse

  • Less common and higher mortality risk
  • Involves whole body
  • Sudden onset skin involvement
32
Q

What is CREST syndrome?

A
  • Calcinosis cutis
  • Raynaud’s phenomenen
  • Esophageal dysmotility
  • Sclerodactyly
  • Telengiectasia
33
Q

What invesitgations are done for systemic sclerosis and what do they show?

A

- Inflammatory markers: normal

- X-Ray hands: calcinosis

- CXR, HRCT, PFT: pulmonary disease

- ECG and ECHO: PA HTN, heart failure, arrhythmias

- ANA: positive

34
Q

How is systemic sclerosis managed?

A
  • No cure
  • CCB/Sildenafil for renal crisis
  • Methotrexate and MMF for skin thickening
  • ACEi to prevent hypertensive crisis
  • Prednisolone for flares
  • PPI for GI symptoms
35
Q

What is Sjogren’s syndrome?

A

Chronic autoimmune disorder that can be primary or secondary.

Characterised by diminished lacrimal and salivary gland secretion due to lymphocytic infiltration and fibrosis of exocrine glands

Usually affects women

36
Q

What are some of the common symptoms and signs of Sjogren’s?

A

MADFRED

  • Myalgia
  • Arthralgia
  • Dry mouth
  • Fatigue
  • Raynaud’s
  • Enlarged parotids
  • Dry eyes and dry mouth
37
Q

What investigations are done to diagnose Sjogren’s?

A

ANA: Anti-Ro and Anti-La usually positive, RF and anti-dsDNA can also be positive

Schirmer’s test: measures conjunctival dryness

Salivary gland biopsy: shows lymphocyte aggregation

38
Q

How is Sjogren’s treated?

A

Symptomatic treatment:

  • Artifical tears
  • Artifical saliva, drink frequently, sugar free sweetd
  • Vaginal lubricants
  • NSAIDs and HCQ can be used for arthralgia
39
Q

What other conditons is Sjogren’s associated with?

A
  • RA
  • SLE
  • Coeliac
  • Primary billiary cirrhosis
  • Autoimmune thyroid disease
40
Q

What malignancy is Sjogren’s associated with?

A

B cell lymphoma

41
Q

What can dermatomyositis be caused by?

A

Paraneoplastic syndrome due to malignancy

42
Q

What is this and what can it indicate?

A

Abnormal nailfold capillaries which is common in SLE and Sjogren’s

43
Q

What autoantibodies are found in SLE?

A

ANA most common

44
Q

What are some examination findings with SLE?

A
  • Low grade fever
  • Pallor
  • Alopecia
  • Arthritis
  • Erythematous rash over face
45
Q

What is a useful mneumonic to remember the diagnostic criteria for SLE?

46
Q

What are some features in SLE that might suggest active disease (a flare up)?

A
  • Low C3/C4
  • Raised inflammatory markers
  • Raised anti-dsDNA
  • Proteinuria
  • Rash, alopecia, fatigue

MEDICINE (25 decks)

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